Transcript What is Reflex Sympathetic Dystrophy Syndrome?

Diagnosis and Treatment
Options of RSD/CRPS
Srinivasa N. Raja, MD
Director of Pain Research
Johns Hopkins University
School of Medicine
Introduction
RSD/CRPS is a chronic neurologic
syndrome characterized by pain of varying
intensity
Early diagnosis and appropriate treatment
are essential to avoid disabling pain
RSD/CRPS is often under-diagnosed and
under-treated by the medical community
What Is Reflex Sympathetic
Dystrophy Syndrome?
Reflex sympathetic dystrophy syndrome
(RSD) is a debilitating neurologic
syndrome characterized by
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Pain and hypersensitivity
Vasomotor skin changes
Functional impairment
Various degrees of trophic change
RSD generally follows a musculoskeletal
trauma
Bogduk N. Current Opinions in Anesthesiology. 2000;14:541-546.
Challenges
Natural course and pathophysiology remain
elusive1
Diagnosis made by exclusion of other causes2
Therapies remain controversial3
Underdiagnosed and undertreated
Significant morbidity and loss of quality of life
1. Jänig W. In: Harden , Baron Janig, eds. Complex regional Pain
Syndrome, Progress in Pain Research and Management. 2001: 3-15.
2. Bogduk N. Current Opinions in Anesthesiology. 2000;14:541-546.
3. Raja SN et al. Anesthesiology. 2002;96:1254-1260.
Terminology: RSD vs CRPS
RSD = traditional term
Complex regional pain syndrome
(CRPS) = more comprehensive term
• Includes disorders not related to sympathetic
nervous system dysfunction
CRPS I = RSD
CRPS II = causalgia (involves nerve injury)
Galer BS et al. In: Loeser, ed. Bonica’s Management of Pain. 2001: 388-411.
Name Change to CRPS
Goals: standardized, reliable diagnostic
criteria and decision rules
• Allow generalization
• Make appropriate treatment selection
• Identify reproducible research samples
Galer BS et al. In: Loeser, ed. Bonica’s Management of Pain. 2001:388-411.
Epidemiology
Age – common in younger adults
• Mean 41.8 years
• Mean age at time of injury 37.7 years
Mean duration of symptoms before pain center
evaluation = 30 months
2.3 to 3 times more frequent in females than males1
Usually involves a single limb in the early stage 2
1. Raja SN et al. Anesthesiology. 2002;96:1254-1260. 2. Galer BS et al. In:
Loeser, ed. Bonica’s Management of Pain. 2001, 388-411.
Clinical Features
Presence of an initiating noxious event or a
cause of immobilization
Continuing pain
• Allodynia: pain from a stimulus that does not
normally provoke pain
• Hyperalgesia: excessive sensitivity to pain
Pain disproportionate to any inciting event
Stanton-Hicks M et al. Pain. 1995;63:127-133. Galer BS et al. In:
Loeser, ed. Bonica’s Management of Pain. 2001; 388-411.
Clinical Features
(cont’d)
History of edema, changes in skin blood
flow, or abnormal sweating in the region of
pain
Exclusion of medical conditions that would
otherwise account for the degree of pain
and dysfunction
Stanton-Hicks M et al. Pain. 1995;63:127-133. Galer BS et al. In: Loeser,
ed. Bonica’s Management of Pain. 2001: 388-411.
Checklist for the Diagnosis of
RSD: History
• Burning pain
• Skin, sensitivity to
touch
• Skin, sensitivity to cold
• Abnormal swelling
• Abnormal hair growth
• Abnormal nail growth
• Abnormal sweating
• Abnormal skin color
changes
• Abnormal skin
temperature changes
• Limited movement
Bogduk N. Current Opinions in Anesthesiology. 2000;14:541-546.
Checklist for the Diagnosis of
RSD/CRPS: Examination
• Mechanical allodynia
• Hyperalgia to single
pinprick
• Summation to multiple
pinprick
• Cold allodynia
• Abnormal swelling
• Abnormal hair growth
• Abnormal skin color
changes
• Abnormal skin
temperature (> or < 1ْ C)
• Limited range of
movement
• Motor neglect
Bogduk N. Current Opinions in Anesthesiology. 2000;14:541-546.
Revised Diagnostic Criteria
Clinical Presentation
Pain and sensory changes disproportionate to the
injury in magnitude or duration
Patients should have at least one symptom in each
of these categories and one sign in 2 or more
categories
 Sensory (hyperesthesia = increased sensitivity to a
sensory stimulation)
 Vasomotor (temperature or skin abnormalities)
 Sudomotor (edema or sweating abnormalities)
 Motor (decreased range of movement, weakness,
tremor, or neglect)
1. Bruehl et al. Pain. 1999;81:147-154. 2. Harden et al. Pain. 1999;83:211219.
Swelling and Color Changes
Abnormal Sweating in RSD
Differential Diagnoses
• Diabetic and smallfiber peripheral
neuropathies
• Entrapment
neuropathies
• Thoracic outlet
syndrome
• Discogenic disease
• Deep vein
thrombosis
• Cellulitis
• Vascular
insufficiency
• Lymphedema
• Erythromelalgia
Raja SN et al. Anesthesiology. 2002;96:1254-1260.
Psychological Aspects
Pain can cause symptoms of psychologic
distress including
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Anxiety
Depression
Fear
Anger
Raja SN et al. Anesthesiology. 2002;96:1254-1260.
Treatment
Goals
• Rehabilitation
• Pain management
• Psychological treatment
Multidisciplinary
• Physiotherapy
• Medical
• Psychological
Stanton-Hicks M et al. Pain Practice. 2002;2:1-16.
Rehabilitation: Clinical
Pathway
Physiotherapy + pain management +
psychological therapies = sequential
progression through the rehabilitation
pathway
PT + OT crucial to patient’s progression
•Therapist assesses patient’s motivation and
helps set goals
•Adequate analgesia, encouragement, and
education of disease process
Stanton-Hicks M et al. Pain Practice. 2002;2:1-16.
Rehabilitation: General Steps
Desensitization of the affected region
Mobilization, edema control, and
isometric strengthening
Stress loading, isotonic strengthening,
range of motion, postural normalization
and aerobic conditioning
Vocational and functional rehabilitation
Stanton-Hicks M et al. Clin J Pain. 1998;14:155-166.
Pharmacalogic Pain
Management
Most drugs used for neuropathic pain are
used to treat RSD/CRPS
• IV alendronate
(bisphosphonate)
• Topic dimethyl
sulfoxide
• Topical clonidine
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IV bretylium
IV ketanserin
IV phentolamine
IV lidocaine
Intranasal calcitonin
Raja SN et al. Anesthesiology. 2002;96:1254-1260.
Kingery WS. Pain.1997;73:123-139
Minimally Invasive Therapies
Sympathetic, IV regional, and somatic nerve
blocks
Patients with a sympathetic component to
their pain (SMP) should receive nerve
blocks
For patients without SMP, a somatic block
or epidural infusion may be indicated to
optimize analgesia for PT
Stanton-Hicks M et al. Pain Practice. 2002;2:1-16.
More Invasive Therapies
Neuroaugmentation
Spinal cord stimulation
Intrathecal drug delivery
Stanton-Hicks M et al. Pain Practice. 2002;2:1-16.
Surgical Therapies:
Sympathectomy
• Controversial procedure
• In carefully selected patients, may result
in reduction in pain severity and disability
Patients with SMP who respond to
selectivesympathetic blockade
• Radiofrequency and neurolytic
techniques are alternatives to a surgical
sympathectomy
Stanton-Hicks M et al. Pain Practice. 2002;2:1-16.
Bandyk DF et al. J Vasc Surg. 2002;35:269-277.
Other Therapies
• Behavioral modification
• Psychiatric consultation
• Complimentary and Alternative therapies
Acupuncture
Raja SN et al. Anesthesiology. 2002; 96:1254-1260.
Prognosis
Difficult to predict
Earlier intervention may be more likely to be
successful
Some patients experience reduced
symptoms or apparently full recovery
Some patients continue to experience
significant disability
Raja SN et al. Anesthesiology. 2002;96:1254-1260.
Conclusions
RSD/CRPS is a chronic neurologic
syndrome
Not all patients have the same set of
symptoms
Early diagnosis and appropriate treatment
is essential
Ideal treatment should be multidisciplinary
Bibliography
Bandyk DF, Johnson BL, Kirkpatrick AF, Novotney ML, Back MR, Schmacht DC.
Surgical sympathectomy for reflex sympathetic dystrophy syndromes. J Vasc
Surg. 2002;35:269-277.
Bogduk N. Complex regional pain syndrome. Current Opinions in Anesthesiology.
2000;14:541-546.
Bruehl SP, Harden RN, Galer BS, et al. External validation of IASP diagnostic
criteria for complex regional pain syndrome and proposed research diagnostic
criteria. Internal Association for the Study of Pain. Pain. 1999;81:147-154.
Galer BS, Schwartz L, Allen RJ. In: Loeser, ed. Bonica’s Management of Pain.
2001: 388-411.
Harden RN, Bruehl SP, Galer BS, et al. Complex regional pain syndrome: are the
IASP diagnostic criteria valid and sufficiently comprehensive? Pain. 1999;83:211219.
Bibliography (continued)
Jänig W. CRPS-I and CRPS-II: A strategic view, In: Harden , Baron Jänig, eds.
Complex regional Pain Syndrome, Progress in Pain Research and Management.
2001: 3-15.
Kingery WS. Pain. A critical review of controlled clinical trials for peripheral
neuropathic pain and complex regional pain syndromes. 1997;73:123-139.
Raja SN , Grabow TS. Complex regional pain syndrome I (Reflex Sympathetic
Dystrophy) Anesthesiology. 2002;96:1254-1260.
Stanton-Hicks M, Burton AW, Bruehl SP, et al. An updated interdisciplinary
clinical pathway for CRPS: Report of an expert panel. Pain Practice. 2002;2:1-16.
Stanton-Hicks M, Jänig W, Hassenbusch S, et al. Reflex sympathetic dystrophy:
changing concepts and taxonomy. Pain. 1995;63:127-133
Stanton-Hicks M, Baron R, Boas R, et al. Complex Regional Pain Syndrome:
guidelines for therapy. Clin J Pain. 1998;14:155-166.